A 37-year-old man developed encephalopathy with prominent eosinophilia. Magnetic resonance imaging (MRI) revealed multiple T2-weighted high signal intensity lesions with dimeglumine gadopentetate (Gd-DTPA) enhancement on Tl-weighted images, which were distributed in the cerebral cortex, thalamus, deep white matter and cerebellum. He was diagnosed as having systemic mastocytosis on the basis ofproliferating mast cells in the bone marrowand peripheral eosinophilia. Following steroid administration, there was a rapid improvementof his symptomsand laboratory data. To our knowledge, this was the first reported case of systemic mastocytosis provoking encephalopathy with serial MRIfindings. (Internal Medicine 33: 23-26, 1994)
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